EDS mainly affects the connective tissue holding the joints together – The connective tissue is looser for people with EDS.
Ehlers-Danlos Syndrome (EDS) is a rare disease occurring in approximately 1 in 5,000 people. EDS can result from a gene mutation that can be inherited, or it can occur without a family history. There are 13 subtypes of EDS, with Classical and Hypermobile-Type being the most common prevalent.
Recommended course: Connective Tissue Disorders of the Ehlers-Danlos Syndromes (EDS)
EDS mainly affects the connective tissue holding the joints together. The connective tissue is looser for people with EDS. This laxity allows the joints to be hypermobile and move far past the normal range of motion. Joint pain, subluxations, and dislocations are common because of this hypermobility.
EDS can affect more than the connective tissue
Various types of EDS cause different signs and symptoms of this disease. Additional symptoms of EDS include:
- Soft, stretchy, thin skin
- Being prone to bruising
- Unusual scarring or unusually long healing times after a cut or small wound
- Joint and muscle pain
- Constantly feeling tired
- Difficulty concentrating
EDS evaluation is multi-faceted, but delayed diagnoses are not uncommon
A comprehensive EDS evaluation can include a combination of:
- Family history
- A physical exam
- Genetic testing
The Beighton Score uses a nine-point scoring system to determine the overall flexibility of the joints. This test assesses the mobility in the following joints:
- Base of both thumbs
- Knuckles of the pinky fingers
It is not uncommon for an EDS diagnosis to take years. Platinum-selling artist Hasley went to multiple doctors after being sick for years before getting an EDS and other diagnoses.
Clients with EDS should engage in exercise activities
Exercise is essential for maintaining a healthy lifestyle, regardless of where a client is on the wellness continuum. To decrease the risk of injury for clients living with EDS, select exercises carefully. Clients should focus on what they can do for physical activity.
Multi-Grammy award winner Billie Eilish has openly discussed EDS’s effect on her body and life. During a magazine interview, Eilish recounted how she spent her teenage years hating herself and being angry at her painful body. Although she has found success in music, she started as a dancer. Unfortunately, a growth plate injury sustained when she was 13 ended her dance ambitions.
EDS can adversely impact functional performance. However, EDS should not prevent most clients from activity engagement. Appropriate exercise recommendations for clients with Ehlers-Danlos include:
- Isometric exercises
- Gentle range of motion exercises that do not move the joints into hyper-extended or hyper-flexed positions
- Low- or no-impact cardio such as water-based activities and walking
Include pain management in an EDS treatment plan
Osteoarthritis is the most common form of arthritis. According to the Centers for Disease Control and Prevention (CDC), the hand is the second most prevalent host for osteoarthritis. Clients with Ehlers-Danlos Syndrome are prone to osteoarthritis at younger ages and often experience chronic pain in more than one area of the body.
Although there is limited research available, case reports suggest positive outcomes for clients who receive therapeutic intervention for pain. Even though clients can successfully engage in daily activities using non-medicinal pain control, they can sometimes experience pain that needs to be managed with medication.
A 2018 limited-participant research study published by the National Institute of Health (NIH) found that patients who received multi-faceted pain intervention significantly improved their pain. The multidisciplinary pain treatment plan for the study participants included:
- Optimizing daily medications
- Cognitive behavioral therapy
- Graded exercises
- Relaxation strategies
Lifestyle and environmental modifications
Living with chronic pain can be as challenging mentally as it is physically. Participating in daily, social, and leisure activities is difficult when a client is in pain or afraid to dislocate a joint. However, clients with Ehlers-Danlos can still engage in meaningful and functional movements with the proper lifestyle and environmental modifications.
Occupational therapists are well equipped to assess clients’ ability to engage in their daily activities. Evaluating the client’s home or work environment will identify where modifications can make life easier. A few modification recommendations include:
- Completing tasks that require more effort earlier in the day
- Including rest breaks during tasks that require repetitive motions
- Reducing heavier loads into smaller, lighter loads
- Placing heavy, frequently used items where they can be retrieved from waist-level
- Using an ergonomic setup in their home or work office
Reducing the workload of the joints
- Adaptive pens and pencils for completing work or leisure tasks
- Enlarged handle utensils for meal prep and meals
- Use backpacks to distribute loads when transporting items from one location to another
- Enlarged-handled combs and brushes for grooming
- Chairs with lateral and back supports
Joint support for Hypermobile Type Ehlers-Danlos
Changes in Joint position can result from years of little or no support for hyperflexible joints when engaged in daily activities. Joint protection is vital to decrease the incidences of joint deformities and slow the progression of joint damage for clients.
Joint supports can include:
- Kinesio taping
In a 2022 study, researchers used a compressive short-sleeved jacket and studied how it affected shoulder stability and rotator muscles. They concluded that the jacket was able to improve shoulder stability in addition to helping to power in shoulder external rotators during high-speed movements in people with hypermobile type EDS.
Clients with EDS can manage their condition with a wellness lifestyle that includes exercise, lifestyle modifications, and adaptive equipment.
The need for lifestyle modifications, professional interventions, and physical support may change as a client ages. However, the effects of this rare disease can be managed to allow clients to live satisfying and productive lives.
This article was written by Tasha Holmes, MOT, OTR/L, BCP.